Abstract:Kawasaki disease is a systemic vasculitis involving small and medium-sized blood vessels, also known as mucocutaneous lymph node syndrome. Kawasaki disease is an acute vascular disease in children. Cardiovascular damage is the most serious complication of Kawasaki disease. The etiology of Kawasaki Disease is still unclear, and the pathogenesis is difficult to explain with a theory. So far, the diagnosis of KD is still an excluded clinical diagnosis. The application of Z value of coronary artery diameter is very important for the diagnosis, management and prognosis assessment of KD. Early high-dose intravenous immunoglobulin(IVIG) combined with aspirin therapy can effectively inhibit the immune inflammatory response, but some children present with IVIG resistance. As Kawasaki disease continues to be studied, new treatments are emerging, including TNF antagonists, methotrexate, clopidogrel, dipyridamole, rituximab, plasmapheresis, and combination of traditional Chinese medicine. This article summarizes the progress in diagnosis and treatment of Kawasaki disease in children.